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The “rescue operation” for patients with cloacal exstrophy and its variants

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Abstract

Introduction

A common error in the initial operative management of patients with cloacal exstrophy is to create an ileostomy leaving the colon defunctionalized and connected to the urinary tract. These patients benefit from a “rescue operation” to give them the best opportunity to be future pull-through candidates.

Methods

Nineteen patients were identified who underwent an inadequate diversion during the newborn period, leaving a distal defunctionalized colon, and required a “rescue operation”. A retrospective review of the medical records of these patients was performed.

Results

A piece of colon was disconnected from the urinary tract, rescued from the pelvis, and incorporated into the fecal stream. The original stoma was closed, and an end colostomy was created. Fifteen patients were females and four were males. The length of rescued colon ranged from 5.5 to 20 cm. Symptoms present before the operation included: hyperchloremic acidosis (6), urinary tract infections (6), failure to thrive (5), sepsis (1), dehydration (1), and TPN dependent (1). There was resolution of these symptoms post-operatively. On follow up, 10 patients still have their colostomies as we are waiting for continued colonic growth, 6 patients had a pull-through after responding to our bowel management program through the stoma, 2 patients have a permanent stoma, and one patient expired.

Conclusion

When patients with cloacal exstrophy, or its variants, receive an ileostomy or proximal colostomy at birth, a rescue operation should be attempted.

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References

  1. Rickham PP (1960) Vesico-intestinal fissure. Arch Dis Child 35:97–102

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  2. Phillips TM (2011) Spectrum of cloacal exstrophy. Semin Pediatr Surg 20:113–118

    Article  PubMed  Google Scholar 

  3. Mathews R, Jeffs RD, Reiner WG et al (1998) Cloacal exstrophy—improving the quality of life: the Johns Hopkins experience. J Urol 160:2452–2456

    Article  CAS  PubMed  Google Scholar 

  4. Soffer SZ, Rosen NG, Hong AR et al (2000) Cloacal exstrophy: a unified management plan. J Pediatr Surg 35:932–937

    Article  CAS  PubMed  Google Scholar 

  5. Sawaya D, Goldstein S, Seetharamaiah R et al (2010) Gastrointestinal ramifications of the cloacal exstrophy complex: a 44 year experience. J Pediatr Surg 45:171–176

    Article  PubMed  Google Scholar 

  6. Lund DP, Hendren WH (2001) Cloacal exstrophy: a 25-year experience with 50 cases. J Pediatr Surg 36:68–75

    Article  CAS  PubMed  Google Scholar 

  7. Bischoff A, Levitt M, Bauer C et al (2009) Treatment of fecal incontinence with a comprehensive bowel management program. J Pediatr Surg 44:1278–1283

    Article  PubMed  Google Scholar 

  8. Bischoff A, Levitt MA, Peña A (2009) Bowel management for the treatment of pediatric fecal incontinence. Pediatr Surg Int 25:1027–1042

    Article  PubMed Central  PubMed  Google Scholar 

  9. Bischoff A, Tovilla M (2010) A practical approach to the management of pediatric fecal incontinence. Semin Pediatr Surg 19:154–159

    Article  PubMed  Google Scholar 

  10. Husmann DA, McLorie GA, Churchill BM et al (1988) Management of the hindgut in cloacal exstrophy: terminal ileostomy versus colostomy. J Pediatr Surg 23:1107–1113

    Article  CAS  PubMed  Google Scholar 

  11. McHoney M, Ransley PG, Duffy P et al (2004) Cloacal exstrophy: morbidity associated with abnormalities of the gastrointestinal tract and spine. J Pediatr Surg 39:1209–1213

    Article  CAS  PubMed  Google Scholar 

  12. Levitt MA, Mak GZ, Falcone RA et al (2008) Cloacal exstrophy—pullthrough or permanent stoma? a review of 53 patients. J Pediatr Surg 43:164–170

    Article  PubMed  Google Scholar 

  13. Mathews R (2011) Achieving urinary continence in cloacal exstrophy. Semin Pediatr Surg 20:126–129

    Article  PubMed  Google Scholar 

  14. Rangel SJ, Lawal TA, Bischoff A et al (2001) The appendix as a conduit for antegrade continence enemas in patients with anorectal malformations: lessons learned from 163 cases treated over 18 years. J Pediatr Surg 46:1236–1242

    Article  Google Scholar 

  15. Chatoorgoon K, Peña A, Lawal T et al (2011) Neoappendicostomy in the management of pediatric fecal incontinence. J Pediatr Surg 46:1243–1249

    Article  PubMed  Google Scholar 

  16. Howell C, Caldamone A, Snyder H et al (1983) Optimal management of cloacal exstrophy. J Pediatr Surg 18:365–369

    Article  CAS  PubMed  Google Scholar 

  17. Taghizadeh A, Qteishat A, Cuckow PM (2009) Restoring hindgut continuity in cloacal exstrophy: a valuable method of optimizing bowel length. Eur J Pediatr Surg 19:141–144

    Article  CAS  PubMed  Google Scholar 

  18. Davidoff AM, Hebra A, Balmer D et al (1996) Management of the gastrointestinal tract and nutrition in patients with cloacal exstrophy. J Pediatr Surg 31:771–773

    Article  CAS  PubMed  Google Scholar 

  19. Stolar CJH, Randolph JG, Flanigan LP (1990) Cloacal exstrophy: individualized management through a staged surgical approach. J Pediatr Surg 25:505–507

    Article  CAS  PubMed  Google Scholar 

  20. Ricketts RR, Woodard JR, Zwiren GT et al (1991) Modern treatment of cloacal exstrophy. J Pediatr Surg 26:444–450

    Article  CAS  PubMed  Google Scholar 

  21. Bischoff A, Levitt MA, Breech L et al (2013) Covered cloacal exstrophy—a poorly recognized condition: hints for a correct diagnosis. J Pediatr Surg 48:2389–2392

    Article  PubMed  Google Scholar 

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Correspondence to Andrea Bischoff.

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Bischoff, A., Brisighelli, G., Levitt, M.A. et al. The “rescue operation” for patients with cloacal exstrophy and its variants. Pediatr Surg Int 30, 723–727 (2014). https://doi.org/10.1007/s00383-014-3512-z

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  • DOI: https://doi.org/10.1007/s00383-014-3512-z

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